著者

Eisaku TERADA Takeo NISHIDA Yuya FUJITA Yohei MAEDA Masaki HAYAMA Masatoshi TAKAGAKI Hajime NAKAMURA Satoru OSHINO Youichi SAITOH Haruhiko KISHIMA

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.10, pp.215-220, 2023-12-31 (Released:2023-07-13)

参考文献数

20

被引用文献数

1

---

We report a case of invasive sphenoid sinus aspergillosis with progressive internal carotid artery (ICA) stenosis and contralateral carotid occlusion that was successfully treated with percutaneous transluminal angioplasty and stenting (PTAS). A 70-year-old man presented with right-sided visual disturbance, ptosis, and left hemiparesis. Magnetic resonance imaging of the head revealed a space-occupying lesion within the sphenoid sinus with infiltration of the bilateral cavernous sinuses, right ICA occlusion, and multiple watershed cerebral infarcts involving the right cerebral hemisphere. The patient was diagnosed with invasive sinus aspergillosis based on transnasal biopsy findings. Despite intensive antifungal therapy using voriconazole, rapidly progressive aspergillosis led to a new stenotic lesion in the left ICA, which increased the risk of bilateral cerebral hypoperfusion. We performed successful PTAS to prevent critical ischemic events. Finally, aspergillosis was controlled with voriconazole treatment, and the patient was discharged. He showed a favorable outcome, with a patent left ICA observed at a 3-year follow-up. PTAS may be feasible in patients with ICA stenosis and invasive sinus aspergillosis.

著者

Ryosuke OTSUJI Toshiyuki AMANO Satoshi MATSUO Yuichiro MIYAMATSU Kenta HARA So TOKUNAGA Akira NAKAMIZO

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.9, pp.151-155, 2022-12-31 (Released:2022-06-15)

参考文献数

28

被引用文献数

1

---

Endovascular embolization of the middle meningeal artery (MMA) has been reported as an effective method for treating chronic subdural hematoma (CSDH); however, its preventive effect on CSDH following craniotomy is unknown. We present a case in which MMA embolization was ineffective in preventing CSDH following craniotomy. A 56-year-old man who complained of diplopia was diagnosed with sphenoid ridge meningioma with a 3-cm diameter. MMA embolization prior to the operation and total surgical removal of the tumor were performed. Two months postoperatively, the patient complained of headache and hemiparesis of the left side. CSDH with a 15-mm thickness and a midline shift was observed. MMA embolization before inflammation may not play a role in preventing CSDH development because MMA embolization is considered effective in CSDH because it is associated with the blood supply of neovessels that are newly formed due to inflammation. Therefore, MMA embolization might not be effective in preventing the occurrence of CSDH following craniotomy.

著者

Takashi IWAMA Katsuhiro MIZUTANI Hajime KUBO Masahiro KATSUMATA Takenori AKIYAMA Masahiro TODA

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.10, pp.125-130, 2023-12-31 (Released:2023-05-17)

参考文献数

11

---

Patients with neurofibromatosis type 1 not only have characteristic skin findings but are also known to have vascular disorders due to vascular vulnerability. A 44-year-old man with previously undiagnosed neurofibromatosis type 1 was brought to the emergency room due to a sudden subcutaneous hematoma with no history of trauma. Angiography revealed extravasation from the parietal branch of the right superficial temporal artery, which was embolized with n-butyl-2-cyanoacrylate. However, the next day, the patient exhibited an increased subcutaneous hematoma, and new extravascular leakage was detected at the frontal branch of the superficial temporal artery, which was also embolized with n-butyl-2-cyanoacrylate. The patient had physical findings characteristic of neurofibromatosis type 1, such as café-au-lait spots, and was subsequently diagnosed with neurofibromatosis type 1. No obvious neurofibroma or any other subcutaneous lesion associated with neurofibromatosis type 1 was identified in the affected area. Massive idiopathic arterial bleeding in the scalp, although infrequent, can be fatal. Neurofibromatosis type 1 should be considered when a subcutaneous scalp hematoma is observed without a history of trauma, even if the facial skin structure appears normal. Neurofibromatosis type 1 is also known to have multiple sources of hemorrhage. Thus, it is important to repeatedly evaluate vascular structures via cerebral angiography, contrast-enhanced computed tomography, and magnetic resonance imaging, if necessary.

著者

Isamu MIURA Shiro HORISAWA Takakazu KAWAMATA Takaomi TAIRA

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.10, pp.99-102, 2023-12-31 (Released:2023-04-10)

参考文献数

16

---

Cervical dystonia, characterized by the involuntary contraction of cervical muscles, is the most common form of adult dystonia. In a patient with intractable cervical dystonia, we carried out a myotomy of the left obliquus capitis inferior and selective peripheral denervation (SPD) of the posterior branches of the C3-C6 spinal nerves based on preoperative 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT). The patient was a 65-year-old, right-handed man with an unremarkable medical history. His head rotated involuntarily to the left. Medication and botulinum toxin injections were ineffective, and surgical treatment was considered. 18F-FDG PET/CT imaging revealed FDG uptake in the left obliquus capitis inferior, right sternocleidomastoideus, and left splenius capitis. Myotomy of the left obliquus capitis inferior and SPD of the posterior branches of the C3-C6 spinal nerves was performed under general anesthesia. During the 6-month follow-up, the patient's Toronto Western Spasmodic Torticollis Rating Scale score improved from 35 to 9. This case shows that preoperative 18F-FDG PET/CT is effective in identifying dystonic muscles and determining the surgical strategy for cervical dystonia.

著者

Kazuo KAKINUMA Keisuke MORIHARA Yoshiteru SHIMODA Nobuko KAWAKAMI Shigenori KANNO Mayuko OTOMO Teiji TOMINAGA Kyoko SUZUKI

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.10, pp.9-14, 2023-12-31 (Released:2023-02-09)

参考文献数

43

---

Idiopathic normal-pressure hydrocephalus (iNPH) is a neurological disorder that typically presents with gait disturbance, cognitive impairment, and urinary incontinence. Although most patients respond to cerebrospinal-fluid shunting, some do not react well because of shunt failure. A 77-year-old female with iNPH underwent ventriculoperitoneal shunt implantation, and her gait impairment, cognitive dysfunction, and urge urinary incontinence improved. However, 3 years after shunting (at the age of 80), her symptoms gradually recurred for 3 months and she did not respond to shunt valve adjustment. Imaging studies revealed that the ventricular catheter detached from the shunt valve and migrated into the cranium. With immediate revision of the ventriculoperitoneal shunt, her gait disturbance, cognitive dysfunction, and urinary incontinence improved. When a patient whose symptoms have been relieved by cerebrospinal-fluid shunting experiences an exacerbation, it is important to suspect shunt failure, even if many years have passed since the surgery. Identifying the position of the catheter is crucial to determine the cause of shunt failure. Prompt shunt surgery for iNPH can be beneficial, even in elderly patients.

著者

Takuya SUEMATSU Tomoaki MURAKAMI Yoshiko SUDO Tatsuya HAGIOKA Yuhei HOSHIKUMA Takamune ACHIHA Takeshi SHIMIZU Shingo TOYOTA Haruhiko KISHIMA

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.9, pp.329-335, 2022-12-31 (Released:2022-09-23)

参考文献数

21

---

Intimal sarcomas (ISAs) are extremely rare malignant tumors that histologically occur in the tunica intima of large blood vessels of the systemic and pulmonary circulation. Herein, we describe a case of an ISA-based neoplastic aneurysm in the middle cerebral artery (MCA) that resulted in a subarachnoid hemorrhage (SAH). The patient presented to our hospital with severe consciousness disturbance (Glasgow Coma Scale E1V1M2) and anisocoria. On admission, computed tomography (CT) showed a diffuse SAH. At 8 months prior, he presented to a previous hospital with hoarseness. Thoracic CT revealed a threatened rupture of the aorta of the arch. After total arch replacement, he had been diagnosed with ISA from the pathological findings of the resected aorta. Thereafter, he had been treated with adjuvant chemotherapy and radiotherapy without any cerebral vascular imaging studies, before admission at our hospital. Angiogram revealed a multilobar fusiform aneurysm on the right MCA. We performed a superficial temporal artery-MCA anastomosis, trapping, and resection of the affected MCA (including the aneurysm), followed by external decompression. Microscopic hematoxylin-eosin staining showed proliferation of atypical spindle-shaped cells with enlarged nuclei in the lumen of the affected MCA. Immunostaining showed CD31 (±), ERG (+), MDM2 (+), CDK4 (+, slightly), SMA (±), MIB-1 index 13.9%, factor VIII (±), and desmin (−). These pathological findings indicated metastasis of the ISA, which formed the neoplastic aneurysm. An ISA can cause a neoplastic cerebral aneurysm. Therefore, once a patient is diagnosed with an ISA, it is necessary to check periodically the cerebral arteries.

著者

Ryota HAGIHARA Makoto ISOZAKI Masamune KIDOGUCHI Hidetaka ARISHIMA Ken-ichiro KIKUTA

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.9, pp.319-322, 2022-12-31 (Released:2022-09-23)

参考文献数

8

---

A 38-year-old woman presented with impaired consciousness and anisocoria due to a subarachnoid hemorrhage and an intracerebral hemorrhage of the left temporal lobe. Examination revealed severe tortuosity in the left middle cerebral artery and three sequential bead-like aneurysms. She underwent a craniotomy in the acute phase to stop rebleeding at the rupture site and remove the intracerebral hematoma. During the chronic phase, endovascular treatment with superselective balloon test occlusion (ssBTO) was performed for the remaining aneurysms. Preoperative ssBTO was useful in evaluating collateral circulation and assessing the curability of the treatment.

著者

Yoshitaka NAGASHIMA Yusuke NISHIMURA Hiroshi ITO Tomoya NISHII Takahiro OYAMA Ryuta SAITO

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.9, pp.295-299, 2022-12-31 (Released:2022-09-15)

参考文献数

20

---

Arachnoiditis ossificans (AO) is a rare disease, wherein ossified lesions in the subarachnoid space obstruct the flow of spinal fluid or compress the spinal cord, thereby causing myelopathy. Here we describe a rare case of AO and discuss the diagnosis and treatment strategies for this disease. A 66-year-old man with a history of subarachnoid hemorrhage presented with gait disturbance and dysuria for 7 months. Spinal magnetic resonance imaging and computed tomography (CT) myelography showed syringomyelia at the T5-T8 level and dorsally tethered spinal cord at the T8-T10 level. Preoperative noncontrast CT was not performed. The patient was diagnosed with adhesive arachnoiditis and underwent arachnoidolysis. However, intraoperative findings showed the presence of ossification lesions on the dorsal surface of the spinal cord, and intraoperative ultrasound (IOU) showed a hyperintense lesion with acoustic shadowing on the dorsal surface of the spinal cord, with limited visibility of the spinal cord. After removal of the lesions, IOU showed untethered and well-decompressed spinal cord and restoration of cerebrospinal fluid pulsation. Based on these findings, the patient was finally diagnosed with AO, which is an extremely rare disease, with an unknown frequency of occurrence. Therefore, all patients with adhesive spinal arachnoiditis require a preoperative noncontrast CT scan to evaluate for ossification lesions. In this case, we were fortunate to be able to treat AO with IOU, which demonstrated specific findings.

著者

Yosuke OKAZAKI Tatsuya SASAKI Kouji KAWAI Kakeru HOSOMOTO Susumu SASADA Takao YASUHARA Tomoyuki AKIYAMA Yoshiyuki HANAOKA Isao DATE

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.9, pp.307-312, 2022-12-31 (Released:2022-09-15)

参考文献数

23

---

Early-onset isolated (DYT1) dystonia is one of the most common forms of primary dystonia in childhood, and deep brain stimulation of the globus pallidus internus (GPi-DBS) is a highly effective treatment for it. However, the effectiveness of GPi-DBS in monozygotic twins with DYT1 dystonia has never been reported globally. Here, we report the cases of monozygotic twins with DYT1 dystonia who were treated using GPi-DBS, and we include a literature review. The younger brother showed an abnormal gait, with external rotation of the right lower leg at 6 years old. The symptoms gradually became so severe that he had difficulty walking on his own at 9 years of age. Treatment with levodopa-carbidopa partially resolved his symptoms, but most of the symptoms remained. Meanwhile, the older brother developed dystonia in both upper limbs at 8 years of age, with gradual symptom progression. At 13 years of age, they were diagnosed with DYT1 dystonia. Bilateral GPi-DBS was performed in both patients at 16 years of age. Their symptoms remarkably improved after surgery. The Burke-Fahn-Marsden dystonia rating scale (BFMDRS) movement score was reduced from 52 to 2 points for the younger brother and from 35 to 1 point for the older brother. Even if monozygotic twins have the same genes, the onset and severity of symptoms might vary in accordance with differences in epigenomic profiles. However, GPi-DBS treatment was very effective for the two cases; thus, we should consider the surgical interventions for each patient.

著者

Hayato TAKEUCHI Yoshinobu TAKAHASHI Seisuke TANIGAWA Takanari OKAMOTO Yoshinori KODAMA Yukiko SHISHIDO-HARA Ema YOSHIOKA Tomoko SHOFUDA Yonehiro KANEMURA Eiichi KONISHI Naoya HASHIMOTO

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.9, pp.199-208, 2022-12-31 (Released:2022-07-08)

参考文献数

26

---

Despite recent signs of progress in diagnostic radiology, it is quite rare that a glioblastoma (GBM) is detected asymptomatically. We describe two patients with asymptomatic nonenhancing GBMs that were not diagnosed with neoplasia at first. The patients had brain scans as medical checkups, and incidentally lesions were detected. In both cases, surgical specimens histopathologically showed no evidence of neoplasia, whereas molecular genetic findings were isocitrate dehydrogenase (IDH) -wildtype, O6-methylguanine-DNA methyltransferase promoter (pMGMT) unmethylated, and telomerase reverse transcriptase (TERT) promoter mutated, which matched to GBM. One patient was observed without adjuvant therapy and the tumor recurred 7 months later. Reoperation was performed, and histopathologically GBM was confirmed with the same molecular diagnosis as the first surgical specimen. Another patient was carefully observed, and chemoradiotherapy was begun 6 months after the operation following the extension of the lesion. Eventually, because of disease progression, both patients deceased. We postulate that in each case, the tumor was not lower-grade glioma but corresponded to the early growth phase of GBM cells. Thus far, cases of malignant transformation from lower-grade glioma or asymptomatic GBM with typical histologic features are reported. Nevertheless, to the best of our knowledge, no such case of nonenhancing, nonhistologically confirmed GBM was reported. We conjecture these cases shed light on the yet unknown natural history of GBM. GBM can take the form of radiological nonenhancing and histological nonneoplastic fashion before typical morphology. Molecular genetic analysis can diagnose atypical preceding GBM, and we recommend early surgical removal and adjuvant treatment.

著者

Yutaka FUCHINOUE Kei UCHINO Sayaka TERAZONO Noyuki HARADA Kosuke KONDO Nobuo SUGO

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.9, pp.231-236, 2022-12-31 (Released:2022-07-27)

参考文献数

21

被引用文献数

2

---

Subependymoma (SE) is a rare, usually asymptomatic, brain tumor predominantly affecting older adults and occurring in the fourth and lateral ventricles. We report a rare case of SE with intratumoral hemorrhage that could be removed by neuroendoscopy. The 81-year-old patient had been followed as an outpatient for 10 years due to an intraventricular tumor. It did not grow over the patient's lengthy follow-up. The patient was transferred to our hospital after he fainted near his home; at the time of admission, he had mild consciousness disturbance, and his Glasgow Coma Scale score was 10 points (E3V3M4). Computed tomography showed intratumoral hemorrhage and slight ventricular enlargement. Magnetic resonance (MR) imaging showed a 4 cm-sized tumor in the anterior horn of the right lateral ventricle. The lesion appeared as a mixed-intensity solid tumor and showed irregular enhancement with gadolinium. The patient underwent neuroendoscopic tumor resection on the 30th day of the patient's hospital stay. A histopathological examination revealed small tumor cells with round nuclei scattered in the glial fibrillary background. Immunostaining was positive for glial fibrillary acidic protein; these findings are consistent with an SE diagnosis. The patient in this study had hypertension and used anticoagulants, risk factors for intratumoral hemorrhage. For intraventricular tumors with bleeding-particularly in older or more physically frail patients-minimally invasive neuroendoscopic surgery should be considered an option for tumor resection.

著者

Natsuki SUGIYAMA Hiroshi HASEGAWA Kentaro KUDO Ryo MIYAHARA Rikizo SAITO Chikashi MARUKI Masaru TAKASE Akihide KONDO Hidenori OISHI

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.9, pp.225-230, 2022-12-31 (Released:2022-07-27)

参考文献数

18

---

There are only a few case reports in which cholesterol crystals were found in the thrombus retrieved by mechanical thrombectomy for cryptogenic stroke, leading to a definitive diagnosis. We herein report a case of aortogenic embolic stroke diagnosed by the presence of rich cholesterol crystals in the retrieved thrombus and review the previously reported cases. A woman in her 80s was transferred as an emergency due to consciousness disturbance, right conjugate deviation, and severe left hemiparesis. Magnetic resonance imaging showed occlusion of the right middle cerebral artery (MCA) and acute infarction in the territory. The MCA was recanalized by thrombectomy using an aspiration catheter and stent retriever, and the symptoms improved. Although the physiological examination did not detect the embolic source during hospitalization, pathological examination of the thrombus revealed atheroma with numerous cholesterol crystal clefts and intermixing of fibrin. Contrast-enhanced computed tomography performed based on the pathological results showed atheromatous lesions in the aortic arch as the embolic source. As a subsequent treatment, medications of a strong statin and an antiplatelet agent were continued, and the patient had no recurrence. The finding that the retrieved thrombus is a simple atheroma containing cholesterol crystals with poor hemocytes suggests embolism due to plaque rupture. Pathological examination of the thrombus obtained by thrombectomy is one of the useful diagnostic approaches for stroke etiology and the determination of its treatment.

著者

Yu NOMURA Masato NARAOKA Nozomi FUJIWARA Shouhei KINOSHITA Keita YANAGIYA Takao SASAKI Ryouta WATANABE Kouta UENO Norihito SHIMAMURA

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.9, pp.165-169, 2022-12-31 (Released:2022-06-15)

参考文献数

18

被引用文献数

1

---

Chronic subdural hematoma (CSDH) typically develops in the supratentorial region in elderly patients. We treated a case of unilateral supratentorial and bilateral infratentorial CSDH, whereby the patient had a coronavirus disease 2019 (COVID-19) infection combined with disseminated intravascular coagulation 2 months earlier. The patient had not experienced any head trauma before the onset of the CSDH. The postoperative course was uneventful, and the patient experienced no neurological deficit. We propose that we should be aware not only of acute ischemic or hemorrhagic diseases after COVID-19 infection but also of chronic subdural hematoma caused by coagulopathy after a COVID-19 infection.

著者

Narihide SHINODA Shogo TAMURA Masafumi MORI Mitsugu NAKAMURA Kazuyoshi KOROSUE Shigeru KOSE

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.9, pp.63-67, 2022-12-31 (Released:2022-04-01)

参考文献数

21

被引用文献数

1

---

Coronavirus disease 2019 (COVID-19) -related intracranial hemorrhage (ICH) is believed to be associated with at least one known risk factor for ICH, such as hypertension, hyperlipidemia, diabetes mellitus, severe pneumonia, or anticoagulation therapy. However, in this study, we report a case of ICH in a 14-year-old boy with mild COVID-19 infection without pneumonia who had no such risk factors. The only abnormal laboratory finding was temporary depletion of vitamin K-dependent coagulation factors. This case indicates that COVID-19 infection may cause simultaneous asymptomatic intracranial microhemorrhages and temporary depletion of vitamin K-dependent coagulation factors. This temporary depletion might transform the intracranial microhemorrhages into symptomatic ICH.

著者

Keiichi TSUJI Atsushi TSUJI Yayoi YOSHIMURA Hiroto KAWANO Ryo FUJISAWA Kazuhiko NOZAKI

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.8, no.1, pp.617-623, 2021 (Released:2021-09-16)

参考文献数

26

被引用文献数

1

---

Brainstem venous congestion due to dural arteriovenous fistula (dAVF) can mimic brainstem glioma and infarction. We report a case of a 56-year-old woman with a transverse-sigmoid sinus (TS) dAVF. On MRI, she presented with brainstem edema that was difficult to distinguish from brainstem glioma and infarction. She was referred to our hospital for mild dysarthria with right hemiparesis and a suspected left pontine glioma. On MRI, contrast enhancement of the lesion was demarcated by the pontine raphe, and the ipsilateral vein of Rosenthal was dilated. Cerebral angiography revealed TS dAVF with an isolated sinus. Transarterial followed by transvenous coil embolization was performed to reduce shunt flow, resulting in symptom improvement and normal findings on MRI and cerebral angiography. Brainstem venous congestion due to TS dAVF is as rare as adult brainstem glioma. Differentiating the above-mentioned three diseases on the basis of diagnostic imaging findings and clinical course is necessary for appropriate and timely treatment.

著者

Junki SOGANO Kenzo KOSUGI Atsushi OKANO Yoshihiro NIHEI Narumi WATANABE Jin NAKAHARA Masahiro TODA

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.10, pp.349-354, 2023-12-31 (Released:2023-12-20)

参考文献数

26

---

In this study, we report on a case of probable sporadic Creutzfeldt-Jakob disease (sCJD) diagnosed after a difficult course of status epilepticus (SE) in a patient with poststroke epilepsy. The patient was admitted with progressive cognitive decline and convulsive SE; therefore, it was initially thought that the patient had developed SE due to nonadherence to antiseizure medication (ASM) use, but despite treatment with ASMs after admission, no improvement was noted in consciousness disturbance or lateralized periodic discharges (LPDs) on electroencephalogram (EEG) examination. After a refractory course, the progression of LPDs to generalized periodic discharges (GPDs) on EEG and abnormal magnetic resonance imaging (MRI) findings met the diagnostic criteria of sCJD. Even if the patient had epilepsy, such as poststroke epilepsy, as in this case, it is essential to consider other underlying causes, including CJD in cases of superrefractory SE.

著者

Reina MIZUNO Atsushi SASAKI Tomonari SUZUKI Jun-ichi ADACHI Mitsuaki SHIRAHATA Ryo NISHIKAWA Kazuhiko MISHIMA

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.10, pp.343-348, 2023-12-31 (Released:2023-12-20)

参考文献数

13

被引用文献数

1

---

A central nervous system (CNS) tumor with BCL-6 co-repressor (BCOR) internal tandem duplication (CNS tumor with BCOR ITD) is a rare tumor classified as an embryonal tumor by the World Health Organization classification (5th edition), and the prognosis is generally poor. A successfully treated case is reported, and its treatment is discussed. A five-year-old boy presented with a one-month history of headache and vomiting. Magnetic resonance imaging showed a well-demarcated, left-frontal tumor without perifocal edema. The patient underwent complete resection without a neurological deficit.Anti-BCOR antibody showed strong immunoreactivity in tumor nuclei, and the tumor was diagnosed as a CNS tumor with BCOR ITD. The patient received craniospinal irradiation (CSI) comprising 23.4 Gy, followed by a boost to the primary site to a total dose of 30.6 Gy in daily fractions of 1.8 Gy.The chemotherapy comprised four cycles of vincristine, cyclophosphamide, and cisplatin with peripheral blood stem cell rescue. The clinical course was uneventful throughout the treatment, the tumor has not recurred for four years, and no neurological impairment was reported. CSI and multiagent chemotherapy were effective for a CNS tumor with BCOR ITD.

著者

Atsuhito TAISHAKU Takayuki OHNO Takashi IWATA Hiromi SHIBATA Motoki ISHIDA Noritaka AIHARA

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.10, pp.327-330, 2023-12-31 (Released:2023-11-29)

参考文献数

18

---

We report a patient with idiopathic intracranial hypertension (IIH) with bilateral transverse sinus occlusion treated by a lumboperitoneal shunt. A 45-year-old woman presented with visual disturbance and chronic headache. Magnetic resonance (MR) venography and three-dimensional computed tomography angiography (3D-CTA) showed occlusion of both transverse sinuses. After the surgery, MR venography and 3D-CTA showed no blockages in both transverse sinuses. This phenomenon reveals that obstruction of the bilateral transverse sinus is a consequence of intracranial hypertension, not just a single cause. Since many patients have an obstruction or stenosis of the venous sinus, there are some reports of endovascular treatment. Sinus reconstruction is usually effective, but it is not effective in some cases. Reversal of sinus occlusion suggests that endovascular therapy for IIH is inadequate in some cases.

著者

Kenta NAKASE Ryosuke MATSUDA Tomoya OKAMOTO Ichiro NAKAGAWA Maiko TAKEDA Katsumi SAKATA Hiroyuki NAKASE

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.10, pp.315-320, 2023-12-31 (Released:2023-11-11)

参考文献数

30

---

Meningeal melanocytomas of the central nervous system, although typically benign, rarely undergo malignant transformations. A 46-year-old man presented with headache and nausea 4 years after gross total resection of a craniovertebral junction meningeal melanocytoma at another hospital. The initial clinical course was previously reported.1) Computed tomography revealed the presence of multiple intracranial mass lesions. Furthermore, magnetic resonance imaging showed multiple intracranial lesions and meningeal dissemination. A biopsy was performed for a circumflex lesion located in the right frontal lobe. Pathological examination showed anaplastic changes and a Ki-67 index of 33%. Based on the pleomorphic changes and high mitotic activity, the patient was diagnosed with primary cerebral malignant melanoma. The patient received four cycles of nivolumab (80 mg) and ipilimumab (165 mg), followed by whole-brain radiotherapy (37.5 Gy). However, the disease progressed after the third cycle. Genome analysis revealed GNAQ Q209P and SF3B1 R625C mutations, but no treatments related to these gene mutations were available. Despite the seven cycles of nivolumab therapy, the patient eventually passed away 9 months after surgery. This case was a rare example of malignant transformation and leptomeningeal melanomatosis in a meningeal melanocytoma. It highlights the importance of careful follow up after gross total resection. Identification of molecular alterations can lead to better detection of melanocytic melanomas with poor prognosis and high risk of recurrence and metastasis. It can also facilitate the development of novel therapeutic options for these patients.

著者

Kyosuke MATSUNAGA Shinjiro FUKAMI Nobuyuki NAKAJIMA Norio ICHIMASU Michihiro KOHNO

出版者

The Japan Neurosurgical Society

雑誌

NMC Case Report Journal (ISSN:21884226)

巻号頁・発行日

vol.10, pp.309-314, 2023-12-31 (Released:2023-11-11)

参考文献数

11

---

Diffuse midline glioma (DMG), H3 K27M-altered, is a tumor with a poor prognosis mainly found in children. An adolescent patient presented with thalamic hemorrhage, which initially could not be diagnosed as DMG by pathological analysis. A neoplasm in the lateral ventricle close to the previous thalamic hemorrhagic lesion was detected 12 months after the hemorrhage. Thus, endoscopic resection was performed, and a diagnosis was made. Gene expression profiling demonstrated mutation in genes, such as H3F3A and FGFR1. FGFR1 mutation was associated with intratumoral hemorrhage in low-grade gliomas and contributed to longer survival than wild-type FGFR1 in DMG H3K27M. Our findings suggest that patients with DMG, H3 K27-altered, with FGFR1 mutation may be predisposed to intratumoral hemorrhaging and/or have a longer survival time than patients without FGFR1 mutation.